Retinoic Acid Syndrome: A Case Report and Review

Background: The treatment of acute promyelocy!c leukemia with all-trans-re!noic acid (ATRA) some!mes results in a syndrome characterized by fever, respiratory distress, weight gain, pleural or pericardial effusion, and pulmonary infiltrates. We report the major clinical and radiologic features of ATRA syndrome. Materials and Methods: In the past, occasional case reports and literature pertaining to ATRA syndrome. The purpose of our report and the literature review is to heighten physicians’ awareness of this syndrome, which o$en manifests as nonspecific clinical and radiographic findings. Results: The e!opathogenesis of the syndrome remain unclear. The Incidence of the syndrome has varied in reports from 5% to 27% and the mortality from 5%-29%. The !me of !me onset of ATRAS varies. The reported median !me to the occurrence of ATRA syndrome is 7-12 days. Chest radiographs show increased cardiothoracic ra!o in 64% of the pa!ents, increased vascular pedicle width in 76%, increased pulmonary blood volume in 82%, ground-glass opacity in 57%, consolida!on in 60%, nodules in 60% of the pa!ents. Pleural effusion is noted in 75% of the pa!ents either unilateral or bilateral effusions. Pulmonary hemorrhage is developed in about 20% of the pa!ents during course of ATRAS. Conclusions: Diagnosis of ATRAS manifesta!ons and immediately star!ng cor!costeroids a$er the diagnosis of ATRAS may be improve the pa!ents’ outcome. Because the radiologic features of ATRAS are nonspecific, it would be impossible to differen!ate one from the other based solely on these features. . Prompt administra!on of steroids is cri!cal, not only when the diagnosis is defini!vely established, but also at the first sign of unexplained dyspnea, fever, weight gain, or pulmonary infiltrates.